2nd Annual Intemational Pulmonary Alveolar Proteinosis Research Conference. This conference will focus on the molecular mechanisms that disrupt lung surfactant homeostasis, alveolar macrophage function and lung host defense in idiopathic pulmonary alveolar proteinosis (PAP), a fatal lung disease affecting men, women and children. The proposed conference "2nd Annual International PAP Research Conference" evolved from an international collaboration initiated at a PAP mini-symposium held at the 98th International Meeting of the American Thoracic Society in Atlanta in 2002. This collaboration, between Drs. Nakata and Trapnell, led to the First International PAP Research Conference held in Fukuoka, Japan in March, 2003. The specific objectives of this conference are to review the recent scientific progress pertaining to the: 1) pathogenesis of PAP in humans and in GM-CSF deficient mice; 2) critical role of GMCSF in surfactant homeostasis and lung host defense; 3) mechanisms by which GM-CSF regulates the terminal differentiation of alveolar macrophages in mice and man; 4) mechanisms by which GM-CSF and PU.1 regulate alveolar macrophage immune functions and the influence of pulmonary innate immunity on adaptive immune responses; 5) progress in translational research to evaluate the effectiveness of GM-CSF immunotherapy for PAP that is currently underway in the United States and Japan; 6) development of a global consensus regarding the best clinical outcomes measures and biomarkers for the longitudinal study on PAP being conducted within the Rare Lung Disease Clinical Research Network (RLD-CRN), which is funded via a cooperative agreement with NIH through ORD/NCRR; and 7) initiation of a global patient registry for PAP to facilitate and speed the rate progress in PAP research related to pathogenesis and therapeutics development and elucidation of the critical role of GM-CSF in the lung in health and disease. An educational conference for PAP patients will be conduced in parallel with this scientific conference with funds from the RLD-CRN. Recent progress in PAP research represents a paradigm of the intersection of basic science, clinical medicine and translational research, which has taken this disease from obscurity to clarity in a decade.